Can patients with cystic fibrosis have a career in the health
service?

Patients with cystic fibrosis (CF) grow up in contact with a wide variety
of health professionals, so it is hardly surprising that some wish to have a
career in the health service. In an attempt to answer the question posed by
the title, this editorial looks at three issues—the infection risks to
the CF healthcare worker; the infection risks to the CF healthcare worker's
patients; and the challenges of a health service career for an individual who
already has to cope with the considerable demands of a CF therapy regimen.

Infection risks to the CF healthcare worker

Patients and a healthcare environment pose a risk to all healthcare
workers, but for workers with CF the risks may be greater.
Box 1 lists the main organisms
that are likely to be of concern. The fact is that cross-infection has become
a nightmare for all patients with CF. At first the fear was just of
colonization with Burkholderia
cepacia1,2,3,
an organism that exists in several different forms, each with its own
pathogenicity. However, it is now known that patients with CF can all too
easily acquire Pseudomonas aeruginosa from other
patients4,5,6.
For the patient not already colonized, a matter of minutes in the company of a
patient who is colonized may be sufficient for cross-infection to occur, and
the worry is even greater in the case of multi-antibiotic resistant strains of
the organism. Staphylococcus aureus has always been a threat to
patients with CF, and the advent of methicillin-resistant strains has
intensified efforts to prevent cross-infection. A common-place strategy is to
cohort patients, and many CF units now run separate ‘Pseudomonas’,
‘Non-pseudomonas’ and ‘Cepacia’ clinics. Although
there is controversy about how far one should take measures to prevent
cross-infection—objective data are scarce—many units make
strenuous efforts to keep CF patients apart from each other: gone are the
outings for patients with CF to Alton Towers, the day trips to Blackpool, the
CF holiday camps. But keeping patients with CF apart at school can be
extremely problematic, and keeping CF siblings apart is well-nigh impossible.
Some units in North America have persuaded their patients to wear a mask
whenever attending hospital; but, as with most other strategies in CF, there
is no objective evidence in favour of this practice.

Against this background, a healthcare environment such as a hospital is a
minefield for the healthcare worker with CF, who is likely to be at far
greater risk of harm from cross-infection than a non-CF healthcare worker.
Plainly some areas in the hospital or community environment are riskier than
others. Probably the most hazardous environment of all is the CF unit—a
miserable conclusion, because this is just the place where having a healthcare
worker with CF could be of particular value. There is a dearth of published
evidence in this whole area, and there are no data on the relative risks of,
say, working in an intensive care unit, a burns unit, an infectious disease
unit or a respiratory unit. Similarly there are no data on the value of
measures to protect the CF healthcare worker, such as wearing masks, gowns or
gloves.

Risks to patients from CF healthcare workers

The risk to patients will depend on the nature of the organisms colonizing
the CF healthcare worker's respiratory tract.
Box 1 lists some of the more
likely pathogens. If the CF individual has no cough, produces no sputum, and
has no pathogenic bacteria in the respiratory tract, then there is no
increased risk to patients. Alas, few individuals with CF have a completely
symptomless respiratory tract. The large majority pose a threat to a greater
or lesser degree, and regrettably there is no objective way to measure the
hazard they present. Some situations are self-evidently very high
risk—for example, a CF patient expectorating S. aureus on a
surgical ward, or a CF patient with P. aeruginosa on a burns
unit—but in most circumstances any risk will be less obvious.

The challenges of a health service career

Many individuals with CF are highly motivated to
work7,8.
In one small study of 15 CF adolescents who were followed up, 10 were working
in professional, semi-professional or clerical jobs, 3 were attending college
and just 2 were unemployed because of
CF9. Not
surprisingly, the most powerful predictors of being able to work are low
disease severity and high
self-esteem9.

A patient with CF who is considering a health service career—or any
other career for that matter—needs information on the duration and
requirements of training, as well as the demands of the career. These factors
must be considered against the need to maintain a therapeutic regimen which
may include daily exercise, twice daily or more physiotherapy, various
medications, and possibly admission to hospital. A patient with CF considering
a health service career must also give thought to the long term, and what will
happen if and when his or her health deteriorates.

Conclusions

This editorial paints a rather bleak and negative picture. The other side
of the coin is that some people with CF have had immensely successful health
service careers, and a diagnosis of CF should not be seen as a complete
barrier to such a career. The individual with CF who is contemplating a career
in the health service needs to consider the issues of risk, and to discuss
these with both a CF physician and a specialist in occupational health. For
the reader who wishes to explore the subject in greater depth, it is one of
several topics covered in this month's supplement to the JRSM, based
on the latest Section of Paediatrics symposium on
CF10.